Pallister-Killian syndrome is a tetrasomy 12p, an extremely rare congenital anomaly syndrome. It is caused by a chromosomal abnormality due to an extra chromosome consisting of two mirror copies of the short arm（“p”）of chromosome 12. Typical presentations include peculiar facial features, sparse hair, hypotonia, and delayed mental development. In this case report, we describe a case of palatoplasty in a 2-year and 10-month-old female with a cleft palate and macroglossia caused by Pallister-Killian syndrome. The patient was intubated due to symptoms of tongue root subsidence at birth and underwent a tracheostomy to correct her airway obstruction at 21 days of age. An examination of the patient’s DNA using blood cells showed a normal karyotype. No further genetic examinations were performed at the parents’ request. She was diagnosed with Pallister-Killian syndrome clinically based on her facial features and other complications. Her airway expanded slowly as she grew up and this was confirmed by radiographs. In addition, she became capable of speaking a little. A palatoplasty was performed. Significant swelling of the tongue and pharyngeal mucosa was noted intraoperatively. The swelling gradually decreased, and she was discharged without complications. The goal of the next stage is to wean her off her tracheostomy.

　　Xeroderma pigmentosum（XP）is an autosomal recessive genetic disease of DNA repair, which may cause severe sun sensitivity and early development of skin cancers such as squamous cell carcinoma, basal cell carcinoma, and malignant melanoma. The incidence of malignant tumors is particularly high in XP Group A patients, who may require repeated surgical treatment. In addition, XP Group A patients often have delayed mental development, which may make postoperative wound management difficult; thus, it is necessary to take these factors into account when deciding on a reconstruction method. In this study, we experienced a case of basal cell carcinoma in the left lower eyelid of a 10-year-old male patient with XP-Group A. Considering the patient’s age and the fact that he was an XP patient, we performed reconstruction using the periosteal anchoring technique and reported an excellent postoperative course.

　　The accessory auricle often occurs in front of the tragus, but it may occur on the posterior surface of the auricle, cheek, or neck. There are multiple case reports of skin masses similar to the accessory auricle in the nostrils, but few studies have reported such masses at the tip of the nose. We report a rare case of accessory auricle and an accessory auricle-like skin mass on the external nose with facial abnormalities such as incomplete midline cleft lip and midline upper lip sinus of the philtrum.
　　Our case may be described as Pai syndrome; however, there is no consensus on diagnostic criteria and the etiology was unknown. Thus, the present case suggests that multiple morphological abnormalities may appear at the same time in a single place or in multiple places. Further research on etiology and embryology is necessary.

　　The curative treatment for arteriovenous malformation（AVM）is complete resection of the arteriovenous shunt. We present a case of radical resection and reconstruction for a large AVM in the head and neck region using the bordering method. A 30-year-old male presented with an enlarged AVM in the right ear (Schobinger stage II). He had received six embolizations and sclerotherapy procedures for right auricular AVM over the past eight years. The AVM became more extensive and started to bleed easily (Schobinger stage III), so we decided to perform a curative resection using the bordering method. The process was divided into three stages ( 1: Division of the AVM and facial nerves, 2 : Complete excision and flap preparation, 3 : Reconstruction). The operative times were 319, 583, and 426 minutes, respectively, and the operative blood loss was 100, 830, and 105 ml respectively. Complete excision and reconstruction were achieved. Twelve months after the surgery, no recurrence was observed. Using the bordering method, the operative time and bleeding per surgery were decreased, reducing the burden on the patient and medical staff. Our case suggests that the bordering method is useful in treating a large AVM in the head and neck region.

　　Three-dimensional（3D）reconstruction of the orbit is challenging. Herein, we report three cases of 3D orbital reconstruction using split-thickness calvarial bone after orbital bone resection during neurosurgery.
　　The frontal bone removed during tumor resection was split into inner and outer plates. The orbital bar was reconstructed using the inner or outer plate with a titanium plate that had been pre-formed using a 3D model as a template. The lateral and superior walls were then reconstructed by placing two bone plates against the curve of the reconstructed orbital rim. We compared and evaluated the eye position and orbital volume using preoperative and postoperative computed tomography and software.
　　All patients showed preoperative ocular protrusion (mean = 4.34 mm). However, the eye position improved postoperatively (mean protrusion = 0.98 mm). The difference in the postoperative orbital volume between the affected and unaffected sides was < 0.5 cm³ in the two cases in which the 3D model was used and －3.47 cm³ in the case in which the 3D model was not used.
　　The smaller change in orbital bone volume using the 3D model suggests that the use of a 3D model was useful in accurately reproducing the 3D structures.